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      Two rare cases of benign struma ovarii with malignant recurrence

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          Abstract

          Summary

          Struma ovarii is an ovarian teratoma that comprises 2–5% of all ovarian teratomas. Malignant transformation of struma ovarii occurs in less than 5% of all cases, and metastatic disease is even rarer. We report two cases initially diagnosed with benign struma ovarii that presented malignant transformation, specifically highly differentiated follicular carcinoma of the ovary (HDFCO), some years after the first diagnosis. Case 1 concerns a 37-year-old female featuring HDFCO of the right ovary with multiple metastatic foci, who was diagnosed with benign struma ovarii 14 years ago. Case 2 concerns a 26-year-old female diagnosed with HDFCO of the left ovary. This patient was initially diagnosed with benign struma ovarii 6 years ago that recurred 4 years after the diagnosis. Both patients were treated with surgery, adjunctive total thyroidectomy, and radioactive iodine ( 131I) therapy.

          Learning points
          • Malignant transformation of struma ovarii is very rare (<5%).

          • Diagnosis of HDFCO without extra ovarian dissemination is difficult due to the resemblance of its histological appearance with normal thyroid tissue.

          • There is no consensus on the postoperative treatment of malignant struma ovarii (MSO). Clinical and histological features of MSO should be assessed for the postoperative treatment decisions.

          • TSH suppression and thyroglobulin level measurements are necessary for patient follow-up.

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          Most cited references13

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          Clinical characteristics of struma ovarii.

          To evaluate the clinical characteristics of struma ovarii. Twenty-five cases of struma ovarii were reviewed retrospectively from June 1994 to April 2007. The presenting clinical, radiologic, and pathologic features of the patients were reviewed. The mean age of the patients in this study was 45.3 years. The majority was of premenopausal status. Sixteen patients had clinical symptoms such as low abdominal pain, palpable abdominal mass and vaginal bleeding. Although one patient had an abnormal thyroid function test, the laboratory findings normalized after operative treatment. CA-125 levels were elevated in 6 cases. Diagnosis by preoperative imaging studies were 8 dermoid cysts, while only 3 cases were diagnosed as struma ovarii. There were 4 cases of malignant struma ovarii, and no patients with recurrent disease. Struma ovarii is a rare tumor. The presented clinical, laboratory and radiological findings of patients are very diverse. The diagnosis was confirmed by pathologic findings. The treatment of benign struma ovarii is surgical resection only. The cases of malignant struma ovarii may need adjuvant treatment, but recurrence is uncommon.
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            Malignant struma ovarii: a case report and analysis of cases reported in the literature with focus on survival and I131 therapy.

            Malignant struma ovarii is a rare type of germ cell tumor that is most often diagnosed postoperatively. The natural history and optimal treatment regimen for the disease are essentially unknown due to the small numbers of published cases. A 32-year-old woman presented with pelvic pain and an ovarian mass that was ultimately treated by total abdominal hysterectomy/bilateral salpingo-oophorectomy. Postoperatively, she was diagnosed with a malignant struma ovarii. The patient was subsequently treated with thyroidectomy and I(131) ablation and is currently disease free. A Medline literature search was performed and clinical data from 23 additional cases were compiled. In this review of 24 cases, 16 patients were followed conservatively postoperatively while 8 received varied additional therapy (4 with I(131)). There were 8 recurrences and all occurred in the conservatively managed patients. I(131) for recurrent disease provided an initial complete response in 7 women. Treatment with thyroidectomy and I(131) should be considered in the first line of management for malignant struma ovarii.
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              Malignant struma ovarii: an analysis of 88 cases, including 27 with extraovarian spread.

              Struma ovarii that display extraovarian spread or later recurrence is exceedingly rare. Among 88 patients with "malignant" struma ovarii followed for prolonged periods, several features helped to predict the adverse clinical course. Adhesions (graded 2 to 4+), peritoneal fluid (> or =1 L) or ovarian serosal rent were worrisome features, occurring in 74% of 27 biologically malignant tumors but only 10% of 61 clinically benign tumors. The size of the strumal component rather than the overall size of the ovarian teratoma also had some predictive value. Tumors with a strumal component or =12 cm were clinically malignant. Except for a papillary pattern or poorly differentiated cancer, no microscopic feature reliably predicted the clinical outcome, including those typically associated with malignancy in primary thyroid tumors. Among the consult cases, 7% with histologic follicular adenomas and 29% with papillary carcinomas were clinically malignant. Unequivocal vascular invasion was rare, precluding assessment of its effect. Optically clear nuclei, when extensive, were useful to diagnose papillary carcinoma, but were present nevertheless in smaller numbers in both macrofollicular and microfollicular adenomas. Eight tumors confined initially to the ovary (stage 1) recurred. Papillary carcinomas recurred earlier (average 4 y) than follicular adenomatous neoplasms (average 11 y, range: 1-29 y). Overall, the survival rate for all patients was 89% at 10 years and 84% at 25 years, indicating the need for routine long-term follow-up.

                Author and article information

                Journal
                Endocrinol Diabetes Metab Case Rep
                Endocrinol Diabetes Metab Case Rep
                EDM
                Endocrinology, Diabetes & Metabolism Case Reports
                Bioscientifica Ltd (Bristol )
                2052-0573
                12 April 2024
                01 April 2024
                : 2024
                : 2
                : 23-0122
                Affiliations
                [1 ]Endocrine Unit and Diabetes Center , Department of Clinical Therapeutics, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
                [2 ]Pathology Department , IASO Hospital, Athens, Greece
                [3 ]Department of Gynecological Oncology , IASO Hospital, Athens, Greece
                Author notes
                Correspondence should be addressed to K Saltiki: saze@ 123456otenet.gr
                Author information
                http://orcid.org/0009-0007-3304-0817
                http://orcid.org/0000-0002-0651-1376
                http://orcid.org/0000-0002-1421-0653
                Article
                EDM23-0122
                10.1530/EDM-23-0122
                11046340
                38614119
                4029bd27-8074-4497-8ed0-95c3b03462c6
                © the author(s)

                This work is licensed under a Creative Commons Attribution 4.0 International License.

                History
                : 26 October 2023
                : 13 March 2024
                Categories
                Adult
                Female
                White
                Greece
                Ovaries
                Endocrine-Related Cancer
                Tumours and Neoplasia
                Gynaecological Endocrinology
                Error in Diagnosis/Pitfalls and Caveats
                Error in Diagnosis/Pitfalls and Caveats

                adult,female,white,greece,ovaries,endocrine-related cancer,tumours and neoplasia,gynaecological endocrinology,error in diagnosis/pitfalls and caveats,april,2024

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